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{{Idiopathic pulmonary fibrosis }}
{{Idiopathic pulmonary fibrosis }}
{{CMG}}
{{CMG}}; {{AE}} {{AEL}}
==Overview==
==Overview==
The mainstay of therapy for idiopathic pulmonary fibrosis is the supportive care measures which include mechanical ventilation, pulmonary rehbilitation, and vaccination against influenza and pneumococcus. Medical treatment as nintedanib and pirfenidone can be administrated to slow the disease progression.
==Medical Therapy==
==Medical Therapy==
There is currently no consensus on the treatment of IPF. Hence, none of what follows should be taken as specific advice regarding therapy, as the latter is a decision that must be made on a case-by-case basis in individual patients.<ref name="Walter">{{cite journal | last=Walter | first=N | coauthors=Collard HR, Talmadge E. King, Jr. | title=Current perspectives on the treatment of idiopathic pulmonary fibrosis | journal=Proceedings of the American Thoracic Society | volume=3 | issue=4 | pages=330–338 | publisher=American Thoracic Society | month=June |year=2006 | url=http://pats.atsjournals.org/cgi/content/full/3/4/330 | pmid=16738197 | accessdate=2008-03-05 }}</ref>
===Supportive care===
*Patients with idiopathic pulmonary fibrosis receive supportive care alongside the medical therapy.<ref name="pmid21471066">{{cite journal| author=Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK et al.| title=An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. | journal=Am J Respir Crit Care Med | year= 2011 | volume= 183 | issue= 6 | pages= 788-824 | pmid=21471066 | doi=10.1164/rccm.2009-040GL | pmc=5450933 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21471066  }}  [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21690586 Review in: Ann Intern Med. 2011 Jun 21;154(12):JC6-8]</ref>
*Supportive care measures include the following:
**Mechanical ventilation: Nearly all the patients with IPF require oxygen supplementation.
**Pulmonary rehabilitation
**Vaccination against possible causes of pulmonary inflammation as influenza and pneumococcal polysacchride capsule vaccines.<ref name="pmid25233284">{{cite journal| author=Tomczyk S, Bennett NM, Stoecker C, Gierke R, Moore MR, Whitney CG et al.| title=Use of 13-valent pneumococcal conjugate vaccine and 23-valent pneumococcal polysaccharide vaccine among adults aged ≥65 years: recommendations of the Advisory Committee on Immunization Practices (ACIP). | journal=MMWR Morb Mortal Wkly Rep | year= 2014 | volume= 63 | issue= 37 | pages= 822-5 | pmid=25233284 | doi= | pmc=5779453 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25233284  }}</ref>


There is a lack of large, randomized placebo-controlled trials of therapy for IPF. Moreover, many of the earlier studies were based on the hypothesis that IPF is an inflammatory disorder, and hence studied anti-inflammatory agents such as [[corticosteroids]]. Another problem has been that studies conducted prior to the more recent classification of idiopathic interstitial pneumonias failed to distinguish IPF/UIP from NSIP in particular. Hence, many patients with arguably more steroid-responsive diseases were included in earlier studies, confounding the interpretation of their results.
=== Medical treatment ===
 
* There is no specific treatment for idiopathic pulmonary fibrosis. However, there are two medications, nintedanib and pirfenidone, that can be administrated in order to slow the disease progression.<ref name="pmid261771832">{{cite journal| author=Raghu G, Rochwerg B, Zhang Y, Garcia CA, Azuma A, Behr J et al.| title=An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline. | journal=Am J Respir Crit Care Med | year= 2015 | volume= 192 | issue= 2 | pages= e3-19 | pmid=26177183 | doi=10.1164/rccm.201506-1063ST | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26177183  }}</ref>
Small early studies demonstrated that the combination of [[prednisone]] with either [[cyclophosphamide]] or [[azathioprine]] over many months had very modest, if any, beneficial effect in IPF, and were associated with substantial adverse effects (predominantly myelotoxicity). Other treatments studied have included [[interferon gamma]]-1b and the antifibrotic agent pirfenidone. While neither drug has been shown to have substantial benefits over time, both are currently being studied in patients with IPF. Finally, the addition of the [[antioxidant]] [[N-acetylcysteine]] to [[prednisone]] and [[azathioprine]] produced a slight benefit in terms of FVC and DLCO over 12 months of follow up. However, the major benefit appeared to be prevention of the myelotoxicity associated with [[azathioprine]].<ref>{{cite journal |last=Demedts |first=Maurits |coauthors=Juergen Behr; Roland Buhl; et al |title=High-dose acetylcysteine in idiopathic pulmonary fibrosis. The IFIGENIA Study |url=http://content.nejm.org/cgi/content/full/353/21/2229 |journal=New England Journal of Medicine |year=2005 |volume=353 |number=21 |pages=2229-2242}}</ref>
* Medical treatment may include the following therapies:
** Preferred regimen (1) : Nintedanib 150 mg PO q12h 
** Preferred regimen (2):
*** Day 1 to 3: Pirfenidone 267 mg PO q8h 
*** Days 8 to 14: Pirfenidone 534 mg PO q8h 
*** Follwoing days: Pirfenidone 801 mg PO q8h
***


==References==
==References==
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[[Category:Pulmonology]]
[[Category:Pulmonology]]
[[Category:Disease]]
[[Category:Medicine]]
[[Category:Up-To-Date]]
[[Category:Primary Care]

Latest revision as of 18:43, 9 April 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]

Overview

The mainstay of therapy for idiopathic pulmonary fibrosis is the supportive care measures which include mechanical ventilation, pulmonary rehbilitation, and vaccination against influenza and pneumococcus. Medical treatment as nintedanib and pirfenidone can be administrated to slow the disease progression.

Medical Therapy

Supportive care

  • Patients with idiopathic pulmonary fibrosis receive supportive care alongside the medical therapy.[1]
  • Supportive care measures include the following:
    • Mechanical ventilation: Nearly all the patients with IPF require oxygen supplementation.
    • Pulmonary rehabilitation
    • Vaccination against possible causes of pulmonary inflammation as influenza and pneumococcal polysacchride capsule vaccines.[2]

Medical treatment

  • There is no specific treatment for idiopathic pulmonary fibrosis. However, there are two medications, nintedanib and pirfenidone, that can be administrated in order to slow the disease progression.[3]
  • Medical treatment may include the following therapies:
    • Preferred regimen (1) : Nintedanib 150 mg PO q12h 
    • Preferred regimen (2):
      • Day 1 to 3: Pirfenidone 267 mg PO q8h 
      • Days 8 to 14: Pirfenidone 534 mg PO q8h 
      • Follwoing days: Pirfenidone 801 mg PO q8h

References

  1. ↑ Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK; et al. (2011). "An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management". Am J Respir Crit Care Med. 183 (6): 788–824. doi:10.1164/rccm.2009-040GL. PMC 5450933. PMID 21471066. Review in: Ann Intern Med. 2011 Jun 21;154(12):JC6-8
  2. ↑ Tomczyk S, Bennett NM, Stoecker C, Gierke R, Moore MR, Whitney CG; et al. (2014). "Use of 13-valent pneumococcal conjugate vaccine and 23-valent pneumococcal polysaccharide vaccine among adults aged ≥65 years: recommendations of the Advisory Committee on Immunization Practices (ACIP)". MMWR Morb Mortal Wkly Rep. 63 (37): 822–5. PMC 5779453. PMID 25233284.
  3. ↑ Raghu G, Rochwerg B, Zhang Y, Garcia CA, Azuma A, Behr J; et al. (2015). "An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline". Am J Respir Crit Care Med. 192 (2): e3–19. doi:10.1164/rccm.201506-1063ST. PMID 26177183.

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